Adult-onset foveomacular pigment epithelial dystrophy - Clinicopathologic correlation of three cases

Purpose: The authors describe the clinicopathologic features of three patie nts with adult onset foveomacular pigment epithelial dystrophy (AOFPED). Methods: The eyes of three patients were studied ophthalmoscopically and by fluorescein angiography, and obtained postmortem and studied by light and electron microscopy. Results: Histopathologic study of the three patient's eyes disclosed centra l loss of the retinal pigment epithelium and photoreceptor cell layer with a moderate number of pigment-containing macrophages present in the subretin al space and outer retina. To either side, the retinal pigment epithelium w as distended with much lipofuscin, Basal laminar and basal linear deposits were present throughout the central area. No discontinuities of Bruch membr ane were present. Conclusion: The findings in the eyes of three patients with AOFPED included marked aging changes that are similar to those seen in age-related macular degeneration. Pigmented cells with lipofuscin in the subretinal space acco unt for the vitelliform appearance.